Rosai-Dorfman病一例及文献分析

目的探讨Rosai-Dorfman病（RDD）的临床表现、诊断、组织特征及治疗。方法研究RDD1例,结合文献回顾分析。结果RDD1例,病理见大量淋巴细胞、浆细胞及组织细胞,并有吞噬淋巴细胞现象,免疫组化S-100强阳性。本病临床表现多样,易误诊。结论RDD是良性的组织细胞增生性疾病,预后主要取决于患者的免疫功能状态、淋巴结受累数目及结外器官受累部位,诊断主要依靠病理及免疫组化,治疗上以综合治疗为主,治疗后应随访。     

多发性皮下Rosai-Dorfman病1例报告并文献复习

目的：探讨多发性皮下Rosai-Dorfman病的临床病理特征、诊断、鉴别诊断。方法：对1例多发性皮下Rosai-Dorfman病进行组织形态学、免疫组化及特殊染色检查,并随访及复习相关文献。结果：肿块分别位于左侧胸壁及右腹股沟区,两处病变组织学上表现相似,呈细胞疏松区和密集区交替分布的结节状结构,细胞疏松区可见淋巴窦样结构,其间有一些体积较大、胞浆丰富淡染的组织细胞,可见胞质内吞噬物和淋巴细胞＂伸入运动＂;细胞密集区散在淋巴滤泡伴较多浆细胞、中性粒细胞等浸润。免疫表型：组织细胞呈S-100、CD163及CD68阳性。结论：多发性皮下Rosai-Dorfman病罕见,确诊主要依靠形态学及免疫组化标记,无论临床及病理均需与纤维组织细胞瘤、Langerhans细胞组织细胞增生症、非特异性肉芽肿、炎性肌纤维母细胞性肿瘤等相鉴别,治疗以手术切除为主,预后较好。     

淋巴结外Rosai-Dorfman病二例报告并文献复习

为了探讨淋巴结外Rosai-Dorfman病的病理学特征、临床表现、诊断、治疗及预后,对2例结外Rosai-Dorf-man病行HE和免疫组织化学染色观察,并对其进行随访。结果显示,光镜下见病灶内有大量含嗜酸性胞质的组织细胞,胞质中可见被吞噬的淋巴细胞、浆细胞或中性粒细胞,S-100和CD68染色( )。治疗方法多样,疗效及预后不同。初步研究结果提示,结外Rosai-Dorfman病是一种少见的组织细胞增生性病变,有一定的病理学特征。由于其临床表现多样,导致诊断较难,治疗效果不尽相同。     

细胞学印片鉴别诊断恶性淋巴瘤

本文报告214例良、恶性淋巴组织疾病病理标本的细胞学印片鉴别诊断恶性淋巴瘤。对淋巴组织良性增生性疾病和恶性淋巴瘤的细胞学诊断标准分别做了描述。标准如下: 良性增生性疾病正常:生发中心细胞包括大核裂细胞和小核裂细胞,周围有成熟淋巴细胞带围绕;核分裂相很少。慢性非特殊性淋巴腺炎:未成熟淋巴细胞增加;核分裂相可见;有大量吞噬细胞。淋巴滤泡增生:生发中心可见成熟及未成熟的淋巴细胞;许多不同成熟程度的免疫母细胞;核分裂相多见。窦组织细胞增多症:窦组织细胞胞浆内含有大量成熟淋巴细胞。免疫母细胞反应:多量的不同成熟程度的免疫母细胞;浆细胞样     

多发性皮下Rosai-Dorfman病1例报告并文献复习

目的:探讨多发性皮下Rosai-Dorfman病的临床病理特征、诊断、鉴别诊断。方法:对1例多发性皮下Rosai-Dorfman病进行组织形态学、免疫组化及特殊染色检查,并随访及复习相关文献。结果:肿块分别位于左侧胸壁及右腹股沟区,两处病变组织学上表现相似,呈细胞疏松区和密集区交替分布的结节状结构,细胞疏松区可见淋巴窦样结构,其间有一些体积较大、胞浆丰富淡染的组织细胞,可见胞质内吞噬物和淋巴细胞"伸入运动";细胞密集区散在淋巴滤泡伴较多浆细胞、中性粒细胞等浸润。免疫表型:组织细胞呈S-100、CD163及CD68阳性。结论:多发性皮下Rosai-Dorfman病罕见,确诊主要依靠形态学及免疫组化标记,无论临床及病理均需与纤维组织细胞瘤、Langerhans细胞组织细胞增生症、非特异性肉芽肿、炎性肌纤维母细胞性肿瘤等相鉴别,治疗以手术切除为主,预后较好。     

Kikuchi淋巴结炎的临床病理学观察

目的：探讨Kikuchi淋巴结炎的病因及临床特征,提高对Kikuchi淋巴结炎的认识,减少误诊。方法：对31例病理确诊为Kikuchi淋巴结炎的病例,结合临床特点和免疫组化进行分析观察。结果：31例中男性14例,女性17例,临床表现均为发热和浅表淋巴结肿大。所有的病例可见核碎片及淋巴细胞凋亡;淋巴结内出现片状或灶状形态多样的组织细胞增生。免疫组化标记CD45、CD68及CD3均为阳性表达。结论：本病病理形态以组织细胞增生为特征,主要是与组织细胞来源的淋巴瘤进行鉴别,免疫组化标记CD68及CD3阳性表达,对本病诊断有很重要的价值。该病属自限性疾病,预后多良好。     

急性儿童系统性EB病毒阳性T细胞淋巴组织增殖性疾病临床病理观察

目的：探讨急性儿童系统性EB病毒阳性T细胞淋巴组织增殖性疾病的发病机制、临床病理特征及鉴别诊断要点,以缩短诊断时间和减少误诊。方法：结合文献分析1例急性儿童系统性EB病毒阳性T细胞淋巴组织增殖性疾病死亡病例的临床症状和体征、病理特征及免疫组织化学、EBER原位杂交、基因克隆重排结果等。结果：急性儿童系统性EB病毒阳性T细胞淋巴组织增殖性疾病临床上主要表现为嗜血综合征,包括发热、淋巴结及肝脾肿大、外周血三系减少,可伴有腹水及胸腔积液,血清EB病毒载量增高、血清铁蛋白明显增高,肝肾功能、凝血、血脂等均异常;骨髓涂片示异型淋巴细胞约占18%,并可见嗜血现象。淋巴结活检示其结构破坏,淋巴滤泡减少,T区明显扩大,可见轻-中度异型淋巴细胞;淋巴窦扩张,组织细胞增生,可见嗜血现象,间质血管增生。免疫组化证实EB病毒感染的细胞毒性T细胞构成病变主体;EBER原位杂交部分淋巴细胞胞核阳性;淋巴结组织标本基因重排示TCR基因发生克隆性重排,患者在发病第27天因多脏器衰竭死亡。结论：急性儿童系统性EB病毒阳性T细胞淋巴组织增殖性疾病是一种系统性病变,部分患者病情急剧恶化死于严重并发症。该病病情多较复杂,且与其它疾病存在重叠或交叉,早期确诊困难,目前证实其中T淋巴细胞增生为克隆性增生,为T细胞淋巴瘤。应提高对其病理认识,并紧密结合临床、检验、免疫表型、基因重排等因素,减少治疗延误。     

无淋巴结肿大的皮肤Rosai Dorfman病1例及文献复习

Rosai Dorfman病(RDD),又称窦组织细胞增生伴巨大淋巴结病,临床少见,易与淋巴系统和皮肤肿瘤混淆。本病可有多种表现形式,但淋巴结病是其基本特点。软组织的RDD少见,多伴有淋巴结病表现。而软组织发病,淋巴管或全身的淋巴结无受累的罕见,诊断也非常困难。近期Young等[1]报道了1     

骨骼Rosai-Dorfman病的诊疗进展

Rosai-Dorfman病，又称窦组织细胞增生伴巨大淋巴结病，是一种罕见的组织细胞病，通常表现为青少年的无痛性双侧颈部淋巴结肿大。Rosai-Dorfman病发病累及骨骼者不到10%，并且多达75%的骨骼Rosai-Dorfman病患者同时存在软组织病灶。颅骨、颌面骨和胫骨是骨骼Rosai-Dorfman病最常见的发病部位。该病临床表现缺乏特异性，诊断主要依靠常规病理学和免疫组织化学染色检查。临床表现主要是局部疼痛和肿胀。影像学上，通常表现为髓内的溶解性病变，有时伴有周围硬化。目前，Rosai-Dorfman病的病因尚不明确，可能涉及潜在的宿主免疫失调、IgG4相关疾病、多种自身免疫性疾病和基因突变等。目前伴有症状的骨骼Rosai-Dorfman病的治疗方案主要取决于具体病灶位置，主要包括手术刮除或切除，其他治疗方案包括激素治疗和化疗等。由于骨骼Rosai-Dorfman病的临床和影像学表现通常提示恶性病变可能，部分患者可能接受比较激进的治疗。全身PET/CT可以用于Rosai-Dorfman病的分期、随访和评估。     

Kikuchi淋巴结炎的临床病理学观察

目的:探讨Kikuchi淋巴结炎的病因及临床特征,提高对Kikuchi淋巴结炎的认识,减少误诊.方法:对31例病理确诊为Kikuchi淋巴结炎的病例,结合临床特点和免疫组化进行分析观察.结果:31例中男性14例,女性17例,临床表现均为发热和浅表淋巴结肿大.所有的病例可见核碎片及淋巴细胞凋亡;淋巴结内出现片状或灶状形态多样的组织细胞增生.免疫组化标记CD45、CD68及CD3均为阳性表达.结论:本病病理形态以组织细胞增生为特征,主要是与组织细胞来源的淋巴瘤进行鉴别,免疫组化标记CD68及CD3阳性表达,对本病诊断有很重要的价值.该病属自限性疾病,预后多良好.     

Rosai-Dorfman disease. Extranodal sinus histiocytosis in three co-existing sites. A case report

A 73-year-old woman presented with mild anterior uveitis, ipsilateral optic neuropathy, and ipsilateral skin nodules. A compressive mass at the level of the orbital apex and sphenoid wing was found on cranial magnetic resonance imaging. Biopsy of the skin nodules revealed histopathologic evidence of sinus histiocytosis with massive lymphadenopathy, or Rosai–Dorfman disease (RDD). Systemic investigations failed to show any massive lymphadenopathy, making this a case of extranodal RDD. This is a salient case in that it proposes three simultaneous and separate sites of involvement by extranodal RDD. It also exemplifies that RDD should be a suspect diagnosis even in the absence of lymphadenopathy.     

Extranodal rosai-dorfman disease with cutaneous and periodontal involvement: a rare presentation

Sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai-Dorfman disease (RDD) was first described as a distinct clinicopathological entity by Rosai and Dorfman in 1969. SHML is commonly characterized by painless cervical lymphadenopathy, and its clinical course is generally benign and self-limiting. This disorder commonly involves the lymph nodes but may secondarily involve the skin. However, purely cutaneous disease without lymphadenopathy or internal organ involvement rarely occurs. In the absence of the massive lymphadenopathy that is characteristic of RDD, the diagnosis of purely cutaneous RDD may be complicated by the rare, nonspecific clinical appearance of skin lesions and the broad histopathological differential diagnosis of this disorder. A high level of suspicion of this disease on the part of the clinician or pathologist is often required. We present a case of cutaneous RDD associated with involvement of periodontal tissue.     

Extranodal manifestation of Rosai Dorfman Disease of the nasopharynx

The aim of presenting this case is to highlight the fact that extranodal manifestation of Rosai Dorfman Disease (RDD) without lymphadenopathy was seen in a teenaged girl with recurrent episodes of epistaxis, due to a lobulated mass in the nasopharynx, which mimicked malignancy. This case is a rare presentation because the extranodal manifestation of RDD in multisystem organs are reported in literature, but the nasopharyngeal manifestation without lymphadenopathy is unique. The clinical presentation and surgical management of the case by endoscopic sinus surgery, the aetiopathology, differential diagnosis and review of literature are described here.     

Attenuation of Social Class and Reproductive Risk Factor Associations for Hodgkin Lymphoma due to Selection Bias in Controls

OBJECTIVE: Hodgkin lymphoma (HL) risk has been linked with higher social class and lower parity, but our prior population-based case-control study in adult women had unexpected null findings for these variables. Because subject participation was 87% for cases but 65% for random digit-dialing (RDD) controls, we examined representativeness of our controls and the impact of detected bias on prior results. METHODS: Using data from RDD enumeration, abbreviated interviews with nonparticipating controls, and the US census, we compared participating and nonparticipating RDD controls across several age groups and then recomputed odds ratios for risk factor associations adjusted for bias. RESULTS: The 325 RDD control participants were younger, more likely to be white, better educated, and of lower birth order and lower parity than the nonparticipants. Adjustment of odds ratios for bias strengthened previously null findings for education and for parity, breast-feeding and miscarriages in young adult women; these latter changes eliminated previously apparent age modification of risks. CONCLUSIONS: Selection bias in female RDD controls resulted from differential participation by socioeconomic factors, varied with age, and produced underestimations of several associations in young women, including reproductive factors. Thus, our prior conclusions of etiologic irrelevance for some study variables may have been inaccurate.     

Evidence of antiangiogenic and antimetastatic activities of the recombinant disintegrin domain of metargidin

Metargidin, a transmembrane protein of the adamalysin family, and integrins, e.g., alpha5beta1 and alphav, are preferentially expressed on endothelial cells on angiogenesis. Furthermore, metargidin interacts with these integrins via its disintegrin domain. In this study, recombinant human disintegrin domain (RDD) was produced in Escherichia coli by subcloning its cDNA into the pGEX-2T vector, and the effect of purified RDD on different steps of angiogenesis was evaluated. At concentrations of 2-10 micro g/ml, RDD exhibited inhibitory activities in a variety of in vitro functional assays, including endothelial cell proliferation and adhesion on the integrin substrates fibronectin, vitronectin, and fibrinogen. RDD (10 micro g/ml) totally abrogated endothelial cell migration and blocked most capillary formation in a three-dimensional fibrin gel. To test RDD efficacy in vivo, the RDD gene inserted into pBi vector containing a tetracycline-inducible promoter was electrotransferred into nude mouse muscle. RDD was successfully synthesized by muscle cells in vivo as shown by immunolabeling and Western blotting. In addition, 78% less MDA-MB-231 tumor growth, associated with strong inhibition of tumor angiogenesis, was observed in athymic mice bearing electrotransferred RDD. Moreover, in the presence of RDD, 74% fewer B16F10 melanoma lung metastases were found in C57BL/6 mice. Taken together, these results identified this RDD as a potent intrinsic inhibitor of angiogenesis, tumor growth, and metastasis, making it a promising tool for use in anticancer treatment.     

Usefulness of oral corticosteroid in Rosai-Dorfman disease

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a benign self-limiting disorder of unknown aetiology, which is frequently mistaken for lymphoma. There is no consensus as to the proper management of this disorder. In the past, potentially harmful treatments, such as antineoplastic drugs, have been advocated. We describe a 25-year-old woman with RDD who has had a remarkably favourable response to oral prednisolone therapy. Our observation strengthens the conclusions of previous case reports as to the effectiveness of corticosteroids in this condition. This article is aimed at creating awareness among clinical oncologists about this pseudolymphomatous disorder and emphasizing the therapeutic role of corticosteroids.     

Radiation recall dermatitis after docetaxel and external beam radiotherapy. Report of two cases and review of the literature]

Radiation recall refers to a tissue reaction produced by a chemotherapeutic agent in a previously irradiated field that would not occur in a nonirradiated field. Docetaxel is a member of the taxane group of antineoplastic agents that cause disruption of cell division by enhancing microtubule assembly and inhibiting tubulin depolymerisation. As well as in breast cancer and lung cancer treatment, its association in a chemoradiation planned treatment becomes frequent and effective. Most of radiation recall dermatitis (RDD) reported in literature concerned paclitaxel or other drugs. We report two particularly striking cases of RDD with docetaxel and radiotherapy. Even if etiology remains undetermined, a number of hypotheses can be formulated. Familiarity with this phenomenon and potential complications of chemotherapy following tumor irradiation may expedite early diagnosis and appropriate lifesaving treatment.